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ABDOMINAL WALL DEFECTS

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					ABDOMINAL WALL DEFECTS


    Dr. Catherine B. Canete
          OMPHALOCOELE


• Anterior abdominal wall defect at the base
  of the umbilical cord with herniation of the
  umbilical contents
Incidence


•   Small omphalocoele 1:5000
•   Large omphalocoele 1:10000
•   Male to female ratio 1:1
•   Pacific Islanders have low risk for
    omphalocoele
Pathophysiology


• Failure of the midgut to return to abdomen
  by the 10th week of gestation
Clinical Findings
• Covered clinical
  defect of the umbilical
  ring
• Defect may vary from
  2-10 cm
• Sac is composed of
  amnion, Wharton’s
  jelly and peritoneum
• 50% have
  accompanying liver,
  spleen, testes/ovary
• >50% have associated
  defects
• Location:
   – Epigastric
   – Central
   – Hypogastric
• Cord attachment is on
  the sac
          GASTROSCHISIS

• Defect of the anterior abdominal wall just
  lateral to the umbilicus
Incidence


•   1:20,000-30,000
•   Sex ratio 1:1
•   10-15% have associated anomalies
•   40% are premature/SGA
Pathophysiology
• Abnormal involution of right umbilical vein
• Rupture of a small omphalocoele
• Failure of migration and fusion of the lateral
  folds of the embryonic disc on the 3rd-4th
  week of gestation
Lateral folds
• Form
  – Lateral abdominal wall
  – Future umbilical ring
Clinical Findings
• Defect to the right of
  an intact umbilical
  cord allowing
  extrusion of
  abdominal content
• Opening  5 cm
• No covering sac
• Bowels often
  thickened, matted and
  edematous
• 10-15% with intestinal
  atresia
           MANAGEMENT
• ABC
• Heat Management
  – Sterile wrap or sterile bowel bag
  – Radiant warmer
• Fluid Management
  – IV bolus 20 ml/kg LR/NS
  – D10¼NS 2-3 maintenance rate
• Nutrition
  – NPO and TPN
• Gastric Distention
  – OG/NG tube
• Infection Control
  – Ampicillin and Gentamicin
• Associated Defects
• Conservative treatment
  – Reduction by squeezing the sac
  – Painting sac with escharotic agent
     • 0.25% Silver nitrate
     • 0.25% Merbromin (Mercurochrome)
• Surgical Management
  – Skin Flaps
  – Primary Closure
  – Staged Closure
     • Staged repair using silo pouch
Skin Flaps
Primary Closure
Staged Closure
        UMBILICAL HERNIA

• Defect in linea alba,
  subcutaneous tissue
  and skin covering the
  protruding bowel
• Frequent in premature
  infants
   PRUNE BELLY SYNDROME

• Thin, flaccid abdominal wall
• Dilation of bladder, ureter and renal
  collecting system
• 1:30,000-50,000
• 95% are male
        BLADDER EXTROPHY

• Defective enfolding of
  caudal folds
• 3.3 in 100,000 births
• Associated with
  prolapsed vagina or
  rectum, epispadias,
  bifid clitoris or penis
    PENTALOGY OF CANTRELL

•   Omphalocoele
•   Anterior diaphragmatic hernia
•   Sternal cleft
•   Ectopia Cordis
•   Intracardiac defect
BECKWITH-WIEDEMANN
    SYNDROME

          • Macrosomia
          • Macroglossia
          • Organomegaly
          • Abdominal wall
            defects
          • Embryonal tumors
                 Omphalocoele Gastroschisis
Incidence        1:6,000-10,000   1:20,000-30,000
Delivery         Vaginal or CS    CS
Covering Sac     Present          Absent
Size of Defect   Small or large   Small
Cord Location    Onto the sac     On abdominal wall

Bowel            Normal           Edematous, matted
               Omphalocoele Gastroschisis
Other Organs   Liver often out      Rare
Prematurity    10-20%               50-60%
IUGR           Less common          Common
NEC            If sac is ruptured   18%
Associated     >50%                 10-15%
Anomalies
Treatment      Often primary        Often staged
Prognosis      20%-70%              70-90%

				
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posted:4/7/2008
language:English
pages:42